Red blood cells are responsible for carrying oxygen to the entire body. All other cell lines are present and seem quantitatively and morphologically normal. Efficacy and longterm outcome of treatment for pure red cell aplasia after allogeneic stem cell transplantation from major aboincompatible donors. Report of a second patient with antibody to erythroblast nuclei and a remission after immunosuppressive therapy. Acquired pure red cell aplasia associated with thymoma is relatively. Bone marrow contains stem cells which develop into the red blood cells that carry oxygen through the body, the white blood cells that fight infections, and the platelets that help with blood clotting. In another case a patient with lamivudineassociated pure red cell aplasia required 15 units of blood over 3 weeks but recovered swiftly after withdrawal of lamivudine 109 a. Dr subotica 4, 1 belgrade, serbia acquired pure red cell aplasia prca is a serious but email. The management of pure red cell aplasia prca continues to challenge clinical investigators because the. We report a patient with aosd who concurrently developed pure red cell aplasia prca. However, cases have been seen in association with systemic lupus erythematosus, chronic lymphocytic leukemia cll, lymphomas, or. Pure red cell aplasia associated with administration of. Pure red cell aplasia prca or erythroblastopenia refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. Acquired pure red cell aplasia prca is a rare condition of profound anemia characterized by a very low reticulocyte count and the virtual absence of erythroid precursors in the bone marrow.
A 72yearold caucasian male, who was started on isoniazid after the. Thymoma patients with pure red cell aplasia prca or hypogammaglobulinemia goods syndrome are rare, whereas those with both prca and goods syndrome are even rarer. Pure red cell aplasia prca or erythroblastopenia refers to a type of anemia affecting the precursors to red blood cells but not to white blood. In patients with congenital hypoplastic anemia the best results have been reported using corticosteroids. Oct 01, 2003 pure red cell aplasia prca is a relatively rare disease although multiple factors are implied in the pathogenesis of its development.
Pure red cell aplasia prca is a rare disorder that presents with anemia secondary to the failure of erythropoiesis. Hematologic manifestations associated with cmv include thrombocytopenia, anemia, and neutropenia. To join, just click on the box in the picture, answer the questions, and we will approve. It has been demonstrated that prca etiology is based on several factors, including antibodies against marrow erythroid cells and erythropoietin. Allergic skin and systemic reactions in a patient with pure red cell aplasia and antierythropoietin antibodies challenged with different epoetins. Pure red cell aplasia prca is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked.
Hematological disorders in aosd vary from a frequently occurring mild anemia and leukocytosis to uncommon lifethreatening involvement of the bone marrow, as manifested by hemophagocytosis and aplastic anemia. Cureus isoniazid induced pure red blood cell aplasia. Pdf pure redcell aplasia as the presenting feature of the. Pure red cell aplasia in patients who are treated for anemia of chronic kidney disease with erythropoiesisstimulating agents such as epoetin was first reported in. Thymoma with pure red cell aplasia and goods syndrome the. Typical bone marrow findings in prca are shown in fig 1. Pure red cell aplasia prca is a type of anemia caused and.
Thymoma and pure red cell aplasia with hypoplasia of. We report a case of pure red cell aplasia prca caused by parvovirus b19 pvb19 infection, which was transmitted through a kidney allograft. Pure red cell aplasia prca free download as powerpoint presentation. Pdf pure red cell aplasia associated with cytomegalovirus. Pure red cell aplasia prca is a rare disorder of blood production in which the bone marrow, the spongy tissue in the center of the bones, fails to function in an adequate manner resulting in anemia. Pure red cell aplasia blood american society of hematology.
The others, including pure red cell aplasia prca, hypogammaglobulinemia goods syndrome, autoim mune disorders and vasculitis, occur in about 510%. There are 2 types of prca caused by autoimmune processes. Parvovirus b19 manifests as chronic pure red cell aplasia in immunocompromised patients. Pure red blood cell aplasia prca is one of the uncommon causes of anemia. In prca, the bone marrow ceases to produce red blood cells. Aplastic anemia aa and pure red cell aplasia prca are two of the various types of immunemediated cytopenias that can be associated with large granular lymphocyte lgl leukemia. Acquired pure red blood cell aplasia is a disorder of erythroid precursors that results in an isolated normocytic anemia. Good syndrome associated with pure red cell aplasia in a patient. Treatment of erythropoietininduced pure red cell aplasia. This leads to symptoms of anemia, including tiredness and pale skin. Jan 26, 2021 idiopathic pure red cell aplasia prca and secondary prca associated with thymoma and large granular lymphocyte leukemia are generally considered to be immunemediated. This is a very rare condition and usually affects adults. Linezolidinduced pure red blood cell aplasia clinical. Pure red cell aplasia has been attributed to lamivudine in a 29yearold woman.
Feb 14, 2011 approach to normocytic anemia pure red cell aplasia check reticulocyte count normocytic anemia increased is there evidence of hemolysis. Pure red cell aplasia has been attributed to dapsone 36 a 75yearold man with type 2 diabetes taking glibenclamide developed granuloma annulare and was given dapsone 100 mgday. Recurrent bacterial infections of the respiratory and urinary tracts continue, and therefore. Pure red cell aplasia prca is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow.
Pure red cell aplasia induced by erythropoiesisstimulating agents. Pure red cell aplasia and lymphoproliferative disorders. Prca patients were associated with thymoma in approximately 8. A group for patients and family members interested in all aspects prca. The condition has been first described by paul kaznelson in 1922. Pure red cell aplasia with the onset of graft versus host. Pure red cell aplasia an overview sciencedirect topics. The diagnosis of prca should be suspected in a patient with a sudden weekly drop in hb of approximately 1 gdl or a weekly transfusion requirement and low reticulocyte count. Aplastic anemia and pure red cell aplasia associated with. Pure red cell aplasiaa rare disease with multiple causes.
Neonatal pure red cell aplasia due to antim adc fetal. Most cases are secondary to various systemic disorders, lymphomas comprising a major, yet. Pure red cell aplasia, cytomegalovirus, epsteinbarr virus introduction pure red cell aplasia prca is a rare hematological condition, characterized by normocytic normochromic anemia and reticulocytopenia, and selective aplasia of erythroid cell line in bone marrow, but without changes in either white blood cell or platelet. Thymoma with pure red cell aplasia and goods syndrome. Citations 7 references 24 figures 2 abstract and figures. Acquired prca may be either a primary disorder or secondary to some other disorder or agent. With picmonic, get your life back by studying less and remembering more.
The association of red cell aplasia and thymoma interested physicians in the 1930s and ultimately led to laboratory studies linking pure red cell aplasia to immune mechanisms, including the early identification of antierythroid precursor cell antibodies by krantz and later characterization of t cells that inhibited erythropoiesis. Nov 14, 2018 hirokawa m, sawada k, fujishima n, et al. It is an autoimmune disease mediated by t lymphocytes andor by an igg antibody against erythroid precursors in the bone marrow. Pure red cell aplasia associated with cytomegalovirus and. Objectivesto examine clinical features, laboratory test results, treatment, and outcome of dogs with pure red cell aplasia prca designretrospective study animals dogs with severe nonregenerative anemia and bone marrow erythroid aplasia proceduresmedical records of dogs determined to have prca on the basis of results of blood and bone marrow analysis between 1996. A slow progressive normocyticnormochromic anemia and reticulocytopenia, without leukopenia and thrombocytopenia in a patient who, except pallor, does not show abnormal findings on physical examination, should arise the suspicion that he has prca. Pure red cell aplasia genetic and rare diseases information. Pdf pure red cell aplasia prca is a rare disorder that presents with anemia secondary to the failure of erythropoiesis. Antibodymediated pure red cell aplasia is a rare syndrome of anemia associated with a low reticulocyte count, an absence of erythroblasts in the bone marrow, resistance to recombinant human. The association of red cell aplasia and thymoma interested physicians in the 1930s and ultimately led to laboratory studies linking pure red cell aplasia to immune mechanisms, including the early identification of antierythroid precursor cell antibodies by krantz and later characterization of t cells. The presentation and clinical course of pure red cell aplasia in 5 patients are. Levels of the hormone erythropoietin that stimulates the bone marrow to produce red blood cells are usually elevated. Longterm outcome of patients with acquired chronic pure red cell aplasia prca following immunosuppressive therapy.
Wish, in primer on kidney diseases fifth edition, 2009 pure red cell aplasia. Isoniazid is a widely used drug for the treatment of tuberculosis all over the world. Pdf cyclosporin therapy for pure red cell aplasia in. Pure red cell aplasia prca is a rare haematological condition that is characterized by severe aregenerative anaemia due to an almost complete cessation of red blood cell production. Acquired pure red cell aplasia prca is characterized by normochromic normocytic anemia, reticulocytopenia and a marked reduction of bone marrow erythroblasts cell and megakaryocytic lineages 15. The diagnosis of pure red cell precursor aplasia prca is made. Apr 01, 2020 pure red cell aplasia prca is a hematological disorder characterized by anemia, reticulocytopenia and selective erythroid cell hypoplasia of bone marrow. Pure red cell aplasia prca is a form of aplastic anemia that is caused by the production of antierythropoietin antibodies induced by administration of exogenous esas.
Acquired pure red cell aplasia prca is a disease characterised by normocytic, normochromic anaemia, low haematocrit. The amounts of white blood cells and platelet remain normal. Azathioprineinduced pure red blood cell aplasia jama. A full blood count was normal, but 4 weeks later his hemoglobin was 3. The diagnosis of prca should be suspected in a patient with a sudden weekly drop in hb of approximately 1 gdl or a weekly transfusion. Pure red cell aplasia prca was first described by kaznelson 1922 as an anemia due to an almost complete cessation of erythropoiesis, but without leukopenia or thrombocytopenia.
Pure red cell aplasia prca is a rare bone marrow failure characterized by a progressive normocytic anemia and reticulocytopenia. Apr 21, 2016 pure red cell aplasia prca is a rare condition that affects the bone marrow. It is known to cause hepatotoxicity, but in rare instances, it can lead to prca. Successful treatment of a patient with a thymoma and pure red. Pure red cell aplasia secondary to pregnancy, characterization of a syndrome. Pure red cell aplasia hematology, ash education program. The management of pure red cell aplasia prca continues to challenge clinical investigators because the pathophysiology is heterogeneous and poorly understood. Dec 02, 2016 pure red cell aplasia prca is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow.
Pure red cell aplasia in patients who are treated for anemia of chronic kidney. Pure red cell aplasia prca is an uncommon disorder in which maturation arrest occurs in the formation of erythrocytes. Acquired primary prca typically affects adults and leads to symptoms of anemia. Pure red cell aplasia cleveland clinic journal of medicine. Pure red cell aplasia as first manifestation of splenic. It is characterized by an absence of red cell precursors reticulocytes in the marrow and a low red blood cell count. Pure red cell aplasia pcra is a rare disorder of erythropoiesis characterized by a selective and severe decrease in erythrocyte precursors in an otherwise normal bone marrow. There are five treatment regimens that have established efficacy for patients with chronic prca. Nov 24, 2016 pure red cell aplasia prca is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Bone marrow examination revealed the diagnosis of pure red cell aplasia.
Pure red cell aplasia is defined by the combination of anemia, reticulocytopenia, and the absence of erythroid precursors on the marrow examination, but the pathophysiologies are diverse. Pdf monoclonal gammopathyassociated pure red cell aplasia. Most cases are secondary to various systemic disorders, lymphomas comprising a major, yet heterogeneous group of primary causes. Here we present the case report of a 70yearold woman with invasive thymoma and simultaneous prca and goods syndrome, who achieved complete prca remission after thymectomy.
Diamondblackfan anemia is a congenital form of prca. Development of pure red cell aplasia by transmission and. Clonal hematopoiesis in adult pure red cell aplasia. Acquired pure red cell aplasia genetic and rare diseases. Pure red cell aplasia prca is a hematologic syndrome characterized by severe normochromic, normocytic anemia. Cyclosporin a treatment of pure red cell aplasia associated with like to make a few comments. Pure red cell aplasia prca occurs when red blood cells fail to fully develop after they are produced in the bone marrow. Pure red cell aplasia prca is a rare bone marrow failure syndrome defined by a progressive normocytic anaemia and reticulocytopenia without. Pure red blood cell aplasia hematology and oncology msd.
The first page of the pdf of this article appears above. The first case of pure red cell aplasia prca associated with thymoma was reported in 1930 by polayes and lederer 1. Acquired pure red cell aplasia is characterized by a decrease in the number of red blood cells produced in the bone marrow. Pure red cell aplasia pure red cell aplasia prca, a disorder first described in 1922 kaznelson, 1922, can be characterized as an anaemia with the almost complete absence of red cell precursors in the bone marrow, but essentially normal granulopoiesis and megakaryopoiesis. Individuals with this disorder are deficient in the number of precursors of red blood cells erythroblasts.
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